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Picture of the Month—Quiz Case

Picture of the Month—Quiz Case A 16-year-old girl presented with a 3-week history of gingival pain and inflammation; mastication exacerbated the pain. She denied having experienced bleeding, rash, or fever. At the age of 1 year, the patient had a developmental hip dislocation that required surgical treatment. Physical examination revealed a generalized tan pigmentation of the skin, micrognathia, microcephaly, the absence of the fifth finger on both hands, and growth retardation (height, 138 cm; weight, 26 kg; below the third percentile). An oral examination revealed prolonged retention of deciduous teeth and noneruption of permanent teeth, patchy brown discoloration of the tongue (Figure 1), and brown gums with smooth surface texture and generalized inflammation (Figure 2). Laboratory studies revealed a hemoglobin level of 8 g/dL (normal range, 11.0-16.0 g/dL [to convert to grams per liter, multiply by 10.0]), a mean corpuscular volume of 100 μm3 or more (to convert to femtoliters, multiply by 1.0), a neutrophil count of 1100/μL (normal range, 2000-8030/μL [to convert to ×109/L, multiply by 0.001]), and a platelet count of 35 000 × 103/μL (normal range, 150 000-300 000 × 103/μL [to convert to ×109/L, multiply by 1.0]). A panoramic dental radiographic scan shows the noneruption of permanent teeth (Figure 3). The bone marrow biopsy confirmed marrow hypoplasia. View LargeDownload Figure 1. Photograph revealing small pigmented areas of the tongue of a 16-year-old girl. View LargeDownload Figure 2. Photograph showing brown abnormalities of the gums of a 16-year-old girl. View LargeDownload Figure 3. Panoramic dental radiographic scan showing the unerupted teeth of a 16-year-old girl. Answer http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Pediatrics & Adolescent Medicine American Medical Association

Picture of the Month—Quiz Case

Abstract

A 16-year-old girl presented with a 3-week history of gingival pain and inflammation; mastication exacerbated the pain. She denied having experienced bleeding, rash, or fever. At the age of 1 year, the patient had a developmental hip dislocation that required surgical treatment. Physical examination revealed a generalized tan pigmentation of the skin, micrognathia, microcephaly, the absence of the fifth finger on both hands, and growth retardation (height, 138 cm; weight, 26 kg; below the...
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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
1072-4710
eISSN
1538-3628
DOI
10.1001/archpediatrics.2012.189a
Publisher site
See Article on Publisher Site

Abstract

A 16-year-old girl presented with a 3-week history of gingival pain and inflammation; mastication exacerbated the pain. She denied having experienced bleeding, rash, or fever. At the age of 1 year, the patient had a developmental hip dislocation that required surgical treatment. Physical examination revealed a generalized tan pigmentation of the skin, micrognathia, microcephaly, the absence of the fifth finger on both hands, and growth retardation (height, 138 cm; weight, 26 kg; below the third percentile). An oral examination revealed prolonged retention of deciduous teeth and noneruption of permanent teeth, patchy brown discoloration of the tongue (Figure 1), and brown gums with smooth surface texture and generalized inflammation (Figure 2). Laboratory studies revealed a hemoglobin level of 8 g/dL (normal range, 11.0-16.0 g/dL [to convert to grams per liter, multiply by 10.0]), a mean corpuscular volume of 100 μm3 or more (to convert to femtoliters, multiply by 1.0), a neutrophil count of 1100/μL (normal range, 2000-8030/μL [to convert to ×109/L, multiply by 0.001]), and a platelet count of 35 000 × 103/μL (normal range, 150 000-300 000 × 103/μL [to convert to ×109/L, multiply by 1.0]). A panoramic dental radiographic scan shows the noneruption of permanent teeth (Figure 3). The bone marrow biopsy confirmed marrow hypoplasia. View LargeDownload Figure 1. Photograph revealing small pigmented areas of the tongue of a 16-year-old girl. View LargeDownload Figure 2. Photograph showing brown abnormalities of the gums of a 16-year-old girl. View LargeDownload Figure 3. Panoramic dental radiographic scan showing the unerupted teeth of a 16-year-old girl. Answer

Journal

Archives of Pediatrics & Adolescent MedicineAmerican Medical Association

Published: Jul 1, 2012

Keywords: inflammation,dental clinics,gingiva,tongue,pain,surgical procedures, operative,unerupted tooth,microcephaly,micrognathism,hemoglobin measurement,mean corpuscular volume analyses,bone marrow biopsy,bone marrow, hypocellular,hip dislocation,exanthema,fever,growth retardation,hemorrhage,abnormal color,physical examination,laboratory techniques and procedures

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